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dc.date.accessioned2019-01-28T21:34:58Z
dc.date.available2019-01-28T21:34:58Z
dc.date.copyright2019en_NZ
dc.identifier.citationNeuroepidemiology 2019; 52: pp. 128–135, doi: https://doi.org/10.1159/000494115
dc.identifier.issn0251-5350en_NZ
dc.identifier.urihttp://hdl.handle.net/10292/12196
dc.description.abstractBackground: Previous epidemiological studies of genetic muscle disorders have relied on medical records to identify cases and may be at risk of selection biases or have focused on selective population groups. Objectives: This study aimed to determine age-standardised prevalence of genetic muscle disorders through a nationwide, epidemiological study across the lifespan using the capture-recapture method. Methods: Adults and children with a confirmed clinical or molecular diagnosis of a genetic muscle disorder, resident in New Zealand on April 1, 2015 were identified using multiple overlapping sources. Genetic muscle disorders included the muscular dystrophies, congenital myopathies, ion channel myopathies, GNE myopathy, and Pompe disease. Prevalence per 100,000 persons by age, sex, disorder, ethnicity and geographical region with 95% CIs was calculated using Poisson distribution. Direct standardisation was applied to age-standardise prevalence to the world population. Completeness of case ascertainment was determined using capture-recapture modelling. Results: Age standardised minimal point prevalence of all genetic muscle disorders was 22.3 per 100,000 (95% CI 19.5–25.6). Prevalence in Europeans of 24.4 per 100,000, (95% CI 21.1–28.3) was twice that observed in NZ’s other 3 main ethnic groups; Māori (12.6 per 100,000, 95% CI 7.8–20.5), Pasifika (11.0 per 100,000, 95% CI 5.4–23.3), and Asian (9.13 per 100,000, 95% CI 5.0–17.8). Crude prevalence of myotonic dystrophy was 3 times higher in Europeans (10.5 per 100,000, 9.4–11.8) than Māori and Pasifika (2.5 per 100,000, 95% CI 1.5–4.2 and 0.7 per 100,000, 95% CI 0.1–2.7 respectively). There were considerable regional variations in prevalence, although there was no significant association with social deprivation. The final capture-recapture model, with the least deviance, estimated the study ascertained 99.2% of diagnosed cases. Conclusions: Ethnic and regional differences in the prevalence of genetic muscle disorders need to be considered in service delivery planning, evaluation, and decision making.
dc.publisherKarger Publishersen_NZ
dc.relation.urihttps://www.karger.com/Article/FullText/494115
dc.rightsThis article is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND). Usage and distribution for commercial purposes as well as any distribution of modified material requires written permission.
dc.subjectPrevalence; Neuromuscular; Muscular dystrophy; Population-based; Epidemiology
dc.titleA Nationwide, Population-based Prevalence Study of Genetic Muscle Disordersen_NZ
dc.typeJournal Article
dc.rights.accessrightsOpenAccessen_NZ
dc.identifier.doi10.1159/000494115en_NZ
dark.contributor.authorTheadom, Aen_NZ
dark.contributor.authorRodrigues, Men_NZ
dark.contributor.authorPoke, Gen_NZ
dark.contributor.authorO'Grady, Gen_NZ
dark.contributor.authorLove, Den_NZ
dark.contributor.authorHammond-Tooke, Gen_NZ
dark.contributor.authorParmar, Pen_NZ
dark.contributor.authorBaker, Ren_NZ
dark.contributor.authorFeigin, Ven_NZ
dark.contributor.authorJones, Ken_NZ
dark.contributor.authorTe Ao, Ben_NZ
dark.contributor.authorRanta, Aen_NZ
dark.contributor.authorRoxburgh, Ren_NZ
dark.contributor.authorMDPrev Research Groupen_NZ
aut.relation.endpage135
aut.relation.startpage128
aut.relation.volume52en_NZ
pubs.elements-id351953
aut.relation.journalNeuroepidemiologyen_NZ


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